Neuroendocrine tumors (NETs) comprise a heterogeneous group of malignancies from cells derived from the neural crest with neuroendocrine differentiation. Neuroblastoma is an embryonal malignancy of the sympathetic nervous system arising from neuroblasts (pluripotent sympathetic cells). It can be sporadic or nonfamilial in origin. Despite the differences in the site of origin, nomenclature, biological behaviour, and functional status, NETs share certain immunohistochemical and ultrastructural features. NETs are relatively rare tumors with an annual incidence of 6 new cases per 100000 inhabitants. It is primarily a tumor of abdominal origin from where it metastasizes to lymph nodes, liver, orbital sites, and central nervous system. Head and neck NETs are uncommon. We report a rare case of neuroblastoma in a 3-year-old child presenting with an unusual large fungating mass in left parotid region.
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